RESUMO
OBJECTIVE: To call pediatrician's attention to an infrequent cause of severe repetitive epistaxis. METHODS: The authors report a case of Bernard-Soulier Syndrome and also make a review of the literature available in MEDLINE from 1970. RESULTS: The authors describe a 3 year-old boy with severe repetitive epistaxis with hemodinamic repercussions, who required many blood and hemoderivates transfusions. Many diagnostic hypotheses had been formulated, until macroplatelets were found. This finding enabled the diagnosis of Bernard-Soulier Syndrome. A maxillary artery embolization was performed, and the bleedings stopped. CONCLUSIONS: Pediatricians should have a high index of suspection in children with severe repetitive nasal bleedings to diagnose uncommon causes of epistaxis, like Bernard-Soulier Syndrome and other trombocytopaties.